How I treat and manage strokes in sickle cell disease

תאריך: 28/05/2015

Neurologic complications are a major cause of morbidity and mortality in sickle
cell disease (SCD). In children with sickle cell anemia, routine use of transcranial
Doppler screening, coupled with regular blood transfusion therapy, has decreased
the prevalence of overt stroke from ∼11% to 1%.  (Blood. 2015;125(22):3401-3410)

BLOOD, 28 MAY 2015 x VOLUME 125, NUMBER 22

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Sickle Cell Disease in Childhood

תאריך: 31/12/2013

KEY POINTS

  • SCD affects the entire body, beginning in very early infancy, and a multidisciplinary team is needed to care for children with SCD.
  • Some forms of SCD-related morbidity, such as overt stroke, are decreasing owing to prognostic and therapeutic advancements.
  • Primary prevention of organ injury is an important focus of current research.
  • Almost all children born with SCD in developed nations now survive to adulthood, but the transition to adult medical care is a high-risk period for death.

Pediatr Clin N Am 60 (2013) 1363–1381 http://dx.doi.org/10.1016/j.pcl.2013.09.006

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Thalassemias

תאריך: 31/12/2013

KEY POINTS

  • The thalassemia syndromes are a heterogeneous group of disorders characterized by variable degrees of hemolysis, chronic anemia, and ineffective erythropoiesis.
  • Because more patients are living longer, disease- and treatment-related complications are becoming more common.
  • Optimal and safe transfusion support, iron chelation, noninvasive iron assessments, and stem cell therapies provide new tools for effective management of thalassemia.

Pediatr Clin N Am 60 (2013) 1383–1391 http://dx.doi.org/10.1016/j.pcl.2013.08.008

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How I treat transfusional iron overload

תאריך: 1/11/2012

Patients with b-thalassemia major (TM) and other refractory anemias requiring regular blood transfusions accumulate iron that damages the liver, endocrine system, and most importantly the heart. The prognosis in TM has improved remarkably over the past 10 years.

BLOOD, 1 NOVEMBER 2012 | VOLUME 120, NUMBER 18 

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How I treat thalassemia

תאריך: 29/09/2011

The purpose of this article is to set forth our approach to diagnosing and managing the
thalassemias, including b-thalassemia intermedia and b-thalassemia major.

BLOOD, 29 SEPTEMBER 2011 | VOLUME 118, NUMBER 13  

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